Solitary plasmacytoma, in contrast to the disseminated neoplastic proliferation of plasma cells with marked infiltration of multiple organ system in multiple myeloma, is plasma cell neoplasm of a single focus occuring either in bone or soft tissue. The association between a solitary plasmacytoma and peripheral neuropathy is rare, and it is a progressive sensorimotorneuropathy, with a raised CSF protein and mixed demyelination and axonal loss in nerve biopsy. Localized radiotherapy indeed proves to be effective of not only arresting the progress of the neuropathy but also allowing a degree of recovery. We experienced a 55-year-old male with a solitary plasmacytoma and peripheral neuropathy confirmed by the radiologic studies, immunohistochemical stain of nasopharyngeal mucosa biopsy and sural nerve biopsy, which has loss of myelinated fiber and axonal degeneration. Until now, the reported cases are very rare in Korea, so we presented a case of solitary plasmacytoma associated with peripheral neuropathy.
Axons ; Biopsy ; Demyelinating Diseases ; Humans ; Korea ; Male ; Middle Aged ; Mucous Membrane ; Multiple Myeloma ; Myelin Sheath ; Neoplasms, Plasma Cell ; Peripheral Nervous System Diseases* ; Plasma Cells ; Plasmacytoma* ; Radiotherapy ; Sural Nerve

The genome of Hantaan virus, the prototype of the hantavirus genus, is composed of three segmented, single stranded negative sense RNA genome. The 5' and 3' termini of the Hantaan virus RNA genome contain noncoding regions (NCRs) that are highly conserved and complementary to form panhandle stuctures. There are some reports that these NCRs seems to control gene expression and viral replication in influenza virus and vesicular stomatitis virus. In this study, we examined whether NCRs in Hantaan virus play a role in expression of the viral nucleocapsid protein (Np) and foreign (luciferase) gene. The 5' and/or 3' NCR-deleted mutants were constructed and analysed. The Np expression of 5' NCR-deleted clone, it showed 40% reduction. To investigate the role of NCR in foreign gene expression, the clones which are replaced ORF of Hantaan viral Np gene with that of luciferase gene were constructed. The results were similar to those of the experiments using Np gene. These results suggest that 3' NCR is more important than 5' NCR in protein expression. To find out a critical region of 3' NCR in more important than 5' NCR in protein expression. To find out a critical region of 3' NCR in protein expression, several clones with a deleted part of 3' NCR were constructed and analyzed. The deletion of the conserved region in 3' NCR showed 20~30% decrease in Np expression. However there were no change in luciferase activities between clones with or without non-conserved region of 3' NCR. These results suggest that the 3' NCR of Hantaan virus S genome, especially conserved region in 3' NCR, plays and important role in the expression of Hantaan viral Np and foreign genes.
Animals ; Clone Cells ; Ecthyma, Contagious ; Gene Expression ; Genome* ; Hantaan virus ; Hantavirus ; Luciferases ; Nucleocapsid Proteins* ; Nucleocapsid* ; Orthomyxoviridae ; RNA ; Vesicular Stomatitis

PURPOSE: Many kinds of substances are produced on vascular endothelial activation. The aim of this study is to confirm an increase in Endothelin-1 (ET-1), the most potent vasoconstrictor, which is produced by endothelial activation, in patients with chronic anal fissure and to infer the relationship between ET-1 and anal fissure chronicity. METHODS: The study groups are divided into three different groups with 30 subjects each. Group 1 is comprised of healthy volunteers, group 2 of chronic anal fissure patients, and Group 3 of patients with higher than 3rd degree hemorrhoids. Blood samples were taken to measure the ET-1 levels in subject's serum and to compare the results with those for the control groups. RESULTS: Among the 90 subjects, 38 were male, and 52 were female. The average age was 36.8. The average ET-1 level marked 1.47 +/- 0.78 pg/mL for male subjects and 1.16 +/- 0.47 pg/mL for female subjects (P = 0.02). The average ET-1 level in the patient groups is as follow: 1.21 +/- 0.44 pg/mL in group 1, 1.46 +/- 0.83 pg/mL in group 2, and 1.20 +/- 0.56 pg/mL in group 3 (P = 0.14). CONCLUSION: Group 2, the chronic anal fissure patient group, showed a higher ET-1 level than groups 1 and 3, the control group and the hemorrhoid patient group, but this difference had no statistical significance.
Endothelin-1 ; Endothelium ; Female ; Fissure in Ano ; Hemorrhoids ; Humans ; Ischemia ; Male

The calcium sensing receptor (CaSR) plays an important role in calcium homeostasis. Activating mutations of CaSR cause autosomal dominant hypocalcemia by affecting parathyroid hormone secretion in parathyroid gland and calcium resorption in kidney. They can also cause a type 5 Bartter syndrome by inhibiting the apical potassium channel in the thick ascending limb of the loop of Henle in the kidney. This study presents a patient who had autosomal dominant hypocalcemia with Bartter syndrome due to an activating mutation Y829C in the transmembrane domain of the CaSR. Symptoms of hypocalcemia occurred 12 days after birth and medication was started immediately. Medullary nephrocalcinosis and basal ganglia calcification were found at 7 years old and at 17 years old. Three hypercalcemic episodes occurred, one at 14 years old and two at 17 years old. The Bartter syndrome was not severe while the serum calcium concentration was controlled, but during hypercalcemic periods, the symptoms of Bartter syndrome were aggravated.
Bartter Syndrome* ; Basal Ganglia ; Calcium ; Extremities ; Homeostasis ; Humans ; Hypocalcemia* ; Kidney ; Loop of Henle ; Nephrocalcinosis ; Parathyroid Glands ; Parathyroid Hormone ; Parturition ; Potassium Channels ; Receptors, Calcium-Sensing*

We report two cases of porphyric peripheral neuropathy in a 19-year-old male with variegate porphyria and in a 39 year-old male with intermittent acute prophyria. Clinically, there were sensory, motor disturbance and autonomic symptoms including decreased sweating, urinary and sphinctor distrubances. Variegate porphyria showed facial diplegia and positive family history inherited by autosomal dominent trait. Intermittent acute porphyria was combined-with SIADH. Both cases were expired due to respiratory failure. Nerve conduction studies were carried out in two cases and both cases showed slow motor, sensory nerve conductlon velocity ,and significant low CMAPs(Compound Muscle Action Potentials). Sural nerve biopsy was carried out in a variegate prophyria compared with one normal control. Decreased large myelinated fibers was found. In nerve fiber teased study. 8.5% of nerve fibers showed axonal degenration and only 2.3% of the segmental demyelination. There findings are suggesting that the porphyric neuro might be the axonal type.and severe neuropathy in a sign of poor prognosis.
Adult ; Axons ; Biopsy ; Demyelinating Diseases ; Humans ; Inappropriate ADH Syndrome ; Male ; Myelin Sheath ; Nerve Fibers ; Neural Conduction ; Peripheral Nervous System Diseases ; Porphyria, Acute Intermittent ; Porphyria, Variegate ; Prognosis ; Respiratory Insufficiency ; Sural Nerve ; Sweat ; Sweating ; Young Adult

PURPOSE: The purpose of this study was to evaluate early outcomes of ALTA (aluminum potassium sulfate and tannic acid, Ziohn(R)) injection compared with those of a submucosal hemorrhoidectomy for the treatment of internal hemorrhoids. METHODS: From September 2008 to April 2009, a total of 50 patients who had internal hemorrhoids (Golliger grade II to IV) were treated by using either ALTA injection (n=25) or a submucosal hemorrhoidectomy (n=25). Outcomes with respect to pain scores, analgesics use, and satisfaction levels of the patients, and complications were compared. RESULTS: The mean number of hemorrhoidal piles was 3.52 in the ALTA injection group and 3.56 in the operation group. The average amount of ALTA injection was 27.34 cc. Pain scores measured at one day and 7 days after the treatment, and the number of analgesics used in the injection group were significantly lower than those in the operation group (P<0.001). However, there was no significant difference in the satisfaction level between two groups. One case of treatment failure was found in the ALTA injection group. There was no difference in complications between the injection group (n=4) and the operation group (n=5) (P=0.725). CONCLUSION: When compared with a submucosal hemorrhoidectomy, ALTA injection showed less post-treatment pain and less analgesics use. Overall complication rates were not different between the two groups. We found the early outcomes of ALTA injection for the treatment of internal hemorrhoids to be comparable to those of surgery. Thus, large-scale and long-term follow-up studies are needed to clarify the proper indications for ALTA injection.
Analgesics ; Follow-Up Studies ; Hemorrhoidectomy ; Hemorrhoids ; Humans ; Potassium ; Sclerotherapy ; Sulfates ; Tannins ; Treatment Failure

PURPOSE: Hemorrhoidal tissues are normal anatomic structures present in every individual, and they act as cushions and are anchored to the internal anal sphincter by a connective tissue system. When the anchoring connective tissues undergo bears degenerative changes, the hemorrhoids not only bulge but also descend into the lumen of the anal canal. The veins also become distended. The previous hemorrhoidectomy methods (excision and ligation methods) tend to remove excessive amounts of hemorrhoidal tissues, possibly causing incontinence or stenosis. This study introduces a modified hemorrhoidectomy method. METHODS: A retrospective study was done with 650 patients (358 males, 292 females) who underwent hemorroidectomies from Jan. 1997 to Jan. 2000. Under saddle-block anesthesia, the patient was placed in a prone jack-knife position. After narrow incisions on the mucosa of the selected pile, a bilateral submucosal dissection was performed. The pedicle was ligated by transfixing sutures 2 or 3 times with 2-0 chromic catgut to lift up the mucosa. RESULTS: The mean operation time per hemorrhoidal pile was 12.7 minutes, and the mean hospital-stay was 4.3 days. Acute and delayed postoperative anal bleeding occurred in 7 (1.1%) and 3 (0.5%) patients, respectively. The symptoms of both subsided spontaneously. Ninety-three (93) patients (14.3%) reguired nelaton catheterization for voiding difficulty, and one patient (0.2%) showed mild anal stenosis. The most frequent complaint was skin-tag formation (148 cases, 22.8%). In 140 cases, the skin tag was removed under local anesthesia. CONCLUSIONS: It is desirable to keep the normal structure of the anal canal by removing as little of the cushions as possible. Our 'lift-up submucosal hemorrhoidectomy' shows good results and is an easy operative method when compared with Parks' original method.
Anal Canal ; Anesthesia ; Anesthesia, Local ; Catgut ; Catheterization ; Catheters ; Connective Tissue ; Constriction, Pathologic ; Hemorrhage ; Hemorrhoidectomy* ; Hemorrhoids ; Humans ; Ligation ; Male ; Mucous Membrane ; Retrospective Studies ; Skin ; Sutures ; Veins

Myokymic discharge is a train of motor unit potentials that occur spontaneously, recur regularly, and sometimes may be associated with clinical myokymia. We report a 39-years-old male with progressive muscle weakness and atrophy of right upper extremity, who revealed focal myokymic discharges in the intrinsic hand muscles where no denervation was evident on EMG study. The cervical CTMM and MRI study showed a large cervical cord tumor at C3-C6 spine level. Postoperative pathological diagnosis of the tumor was anaplastic astrocytoma.
Astrocytoma ; Atrophy ; Denervation ; Diagnosis ; Hand* ; Humans ; Magnetic Resonance Imaging ; Male ; Muscle Weakness ; Muscles* ; Myokymia ; Spine ; Upper Extremity

A discription is given of 24 patients with the benigen focal amyotrophy; Nineteen had upper-limb and five had lower-limb involvement. The characteristic clinca! Features were insidious onset in the second and third decades, male predominance. Sporadic occurance, wasting and weakness confined to one limb, relatively good prognosis and absence of involvement of the cranial nerves, cerebrum, brainstem, and sensory syetem. The nerve conduction studies, electromyographic pattems, somatosensory evoked potential studies, and CTMM or MR findings suggested anterior horn cell disorders. In nine cases we were able to demonstrate focal atrophy in the lower cervical cord limited to the ipsilateral anterior horn region, and two cases segmental cord atrophy in the lower cervical cord in MR or CTMM studies. The benign focal amyotrophy, for which no causes been described, is benign and self-limiting, unlike most motor neuron diseases.
Animals ; Anterior Horn Cells ; Atrophy ; Brain Stem ; Cerebrum ; Cranial Nerves ; Evoked Potentials, Somatosensory ; Extremities ; Horns ; Humans ; Male ; Motor Neuron Disease ; Neural Conduction ; Prognosis

A discription is given of 24 patients with the benigen focal amyotrophy; Nineteen had upper-limb and five had lower-limb involvement. The characteristic clinca! Features were insidious onset in the second and third decades, male predominance. Sporadic occurance, wasting and weakness confined to one limb, relatively good prognosis and absence of involvement of the cranial nerves, cerebrum, brainstem, and sensory syetem. The nerve conduction studies, electromyographic pattems, somatosensory evoked potential studies, and CTMM or MR findings suggested anterior horn cell disorders. In nine cases we were able to demonstrate focal atrophy in the lower cervical cord limited to the ipsilateral anterior horn region, and two cases segmental cord atrophy in the lower cervical cord in MR or CTMM studies. The benign focal amyotrophy, for which no causes been described, is benign and self-limiting, unlike most motor neuron diseases.
Animals ; Anterior Horn Cells ; Atrophy ; Brain Stem ; Cerebrum ; Cranial Nerves ; Evoked Potentials, Somatosensory ; Extremities ; Horns ; Humans ; Male ; Motor Neuron Disease ; Neural Conduction ; Prognosis